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At least 1,189 records · Page 66Linked to original sources
PubMed1998

Evaluation of benzalkonium chloride chemoneurolytic proximal gastric vagotomy.

BACKGROUND: Transmucosal chemoneurolytic injection of benzalkonium chloride (BAC) has previously been shown to duplicate operative proximal gastric vagotomy (PGV) in controlling gastric acid secretion. In this study, BAC was evaluated as to efficacious dose, methods of delivery, and systemic toxicities. METHODS: Sham celiotomy, operative PGV controls, transmucosal injections through a gastrotomy, and transserosal injections of BAC (saline controls, 0. 625, 1.25, 2.5, 5.0, 10 mg BAC/kg body wt) were administered to Sprague-Dawley rats. After 3 months the rats underwent Congo red testing (CRT), horseradish peroxidase (HRP) neuronal staining, and necropsy. The color density change of the gastric mucosa from basic to acidic demonstrated by the CRT at the time of necropsy was used to calculate the residual anatomic acid-secreting area. Prior to necropsy, subserosal HRP injections into the anterior and posterior stomach walls assayed vagal neuronal viability via retrograde axonal flow. Results were compared by an ANOVA. RESULTS: The results demonstrated that 1.25-10 mg/kg transmucosal BAC replicated the results of operative PGV; 2.5 mg/kg was found to be the most effective dose. All injection groups including saline controls demonstrated similar diminished vagal retrograde axonal flow by HRP testing consistent with local BAC chemoneurolytic effects. No systemic toxic symptoms were observed after tail vein intravenous BAC 1.25, 2.5, and 5.0 mg/kg. CONCLUSIONS: These efficacy studies have demonstrated BAC's potential utility in the performance of endoscopic transmucosal chemoneurolytic PGV.

Animals↗
PubMed2006

Renal amyloidosis in a child with sickle cell anemia.

The kidney is frequently affected in patients with sickle cell syndrome, i.e., homozygous and heterozygous patients, with a consequently large spectrum of renal abnormalities that may range from minimal functional changes to chronic renal failure. Here, we present a 13-year-old boy with sickle cell anemia (SCA) (HbSS) who was referred to our unit with nephrotic syndrome. Renal biopsy revealed AA type amyloidosis on the basis of light microscopic findings, indicating Congo red staining and immunohistochemistry. He had neither a family history of familial Mediterranean fever (FMF) nor any complaint of recurrent abdominal pain, arthritis, and fever, but frequent painful vaso-occlusive crises. The patient was found to have no MEFV gene (Mediterranean feVer) mutations either. Painful episodic attacks might provoke recurrent acute inflammation, leading to repeated stimulation of acute phase responses and cause secondary amyloidosis. To our knowledge, this boy is the first case of SCA complicated by renal amyloidosis observed in childhood.

Adolescent↗
PubMed2004

Histological analysis of the ligamentum flavum of patients with dialysis-related spondyloarthropathy.

Dialysis-related spondyloarthropathy (DRS) is a severe complication of long-term hemodialysis that ultimately leads to functional disability of the upper and lower extremities. Although the cause of this disease is still unknown, it is thought that amyloid deposits are involved. beta2-Microglobulin (beta2M) is a major component of amyloid fibrils, some of which are modified with the advanced glycation end-product (AGE). To clarify the pathophysiology of DRS we histologically examined the ligamentum flavum of the cervical spine in 15 patients with DRS. The mean duration of hemodialysis was 20 years (12-27 years). In addition to the congo red stain for amyloid, beta2M and AGE were detected by immunohistochemical methods. Macrophages were stained with CD68 antibody. Amyloid deposits were found in tissues, although the extent of the stained area differed among the patients. Part of the amyloid deposit area was positively immunostained for beta2M and AGE. In 10 cases macrophages positive for CD68 infiltrated around the amyloid deposits. Comparing these histological findings with the dialysis duration, more positive staining areas for beta2M and AGE were found in the tissue from patients with long-term dialysis. These findings suggest that both beta2M and AGE play roles in the pathogenesis of DRS.

Adult↗
PubMed2005

Liver transplantation and new therapeutic approaches for familial amyloidotic polyneuropathy (FAP).

Liver transplantation has been considered as a promising therapy to halt the progression of clinical symptoms in familial amyloidotic polyneuropathy (FAP) because most transthyretin (TTR) is produced by the liver. In addition, domino liver transplantation using an FAP patient's liver has been performed because of a shortage of donor livers. However, because the use of liver transplantation as therapy for FAP has given rise to several problems, an alternative treatment is needed. We have tried several other approaches. Recent studies suggested that certain metal ions affect amyloidogenesis. Among metal ions tested in an in vitro amyloid formation study, Cr3+ increased stability of both normal and mutant TTR tetramers and suppressed TTR amyloidogenesis induced by low pH. Our findings indicate that Cr3+ acts to suppress TTR amyloidogenesis. BSB, a Congo red derivative that binds to amyloid fibrils in FAP as well as to those in senile plaques in Alzheimer's disease, effectively suppressed TTR amyloid formation in vitro. BSB may thus be useful for preventing amyloid formation. Free radical scavenger therapy was also tried in FAP patients but yielded no conclusive results. Immunization for transgenic mice having the ATTR V30M gene using ATTR Y78P resulted in suppression of amyloid deposits. Finally, an RNA/DNA chimera and single-stranded oligonucleotides (SSOs) were tested in vitro and in vivo in an attempt to repair the amyloidogenic TTR gene in the liver and retina. On the basis of results achieved so far, SSO is a promising tool for gene therapy.

Amyloid↗
PubMed2004

The prevalence of subclinical amyloidosis in Polish patients with rheumatoid arthritis.

The aims of this study were to determine the proportion of rheumatoid arthritis (RA) patients attending hospital in whom amyloid deposits were present in abdominal fat aspiration (AFA) samples, and to assess possible risk factors for amyloid development in RA. One -hundred and twenty-one patients (16 males, 105 females) with RA referred to the Department of Rheumatology in Wroclaw between 1996 and 2001 were studied regardless of RA duration or laboratory findings. Abdominal subcutaneous fine-needle aspiration was performed, and samples of adipose tissue stained with alkaline Congo red then examined by polarized light microscopy. The presence or absence of amyloid fat deposits (AFD) was determined according to whether typical apple-green birefringence was observed. Amyloid deposits were found in 35 (29%) patients. Amyloidosis was significantly more common in males and in patients with longer disease duration. Patients with AFD had previously undergone less treatment with disease-modifying antirheumatic drugs (DMARDs) than those without AFD, and significantly fewer patients with AFD had previously taken methotrexate than those without AFD (25% vs 45%; p<0.01). Renal involvement was found in 12 of 35 patients with AFD (34%). Using the AFA technique, amyloid deposits were found commonly in RA patients, particularly in males with longer disease duration and in patients not treated intensively with DMARDs, especially methotrexate. AFA has potential useful application as a method for detecting amyloidosis before the overt occurrence of renal or other pathology related to amyloid deposits.

Abdomen↗
PubMed2006

Analysis of different genetic traits and their association with biofilm formation in Staphylococcus epidermidis isolates from central venous catheter infections.

The aim of the present study was to characterize clinical isolates of Staphylococcus epidermidis, one of the bacterial species most often implicated in foreign-body-associated infections, for their ability to form biofilms and for the presence of mecA and IS256 element. Sixty-seven Staphylococcus epidermidis clinical isolates, obtained from implantable medical devices, were investigated. Overall, 70% of the strains were positive for ica operon genes, 85% possessed atlE, and 46% contained aap. In 89% of the population, the Congo red agar test confirmed the correlation between the presence of ica genes and slime expression. Almost all of the strains could be classified as biofilm producers by both the crystal violet assay and microscopy. The bacterial population studied showed a very high frequency of strains positive for mecA as well as for the IS256 element. Although well-structured biofilms have been previously observed only in those strains possessing genes belonging to the ica operon, this study demonstrates that strains lacking specific biofilm-formation determinants can be isolated from catheters and can form a biofilm in vitro. Hence, different and yet-to-be identified factors may work together in the formation and organization of complex staphylococcal microbial communities and sustain infections associated with implanted medical devices.

Anti-Bacterial Agents↗
PubMed2005

Fibrillary glomerulonephritis associated with monoclonal gammopathy of undetermined significance showing lambda-type Bence Jones protein.

A 79-year-old woman was admitted to our hospital because of leg edema due to a nephrotic syndrome. Urinary and serum immunoelectrophoresis showed positive for the lambda type of Bence Jones protein. A bone marrow aspiration test revealed mild plasmacytosis (6.4% of the total cells). These findings confirmed her diagnosis of monoclonal gammopathy of undetermined significance (MGUS). Her renal biopsy specimen revealed mild mesangial cell proliferation and an increase in the mesangial matrix. Immunofluorescence studies showed positive staining for IgG, IgA, C3, and kappa and lambda light chains in the capillary wall and mesangium area. Electron microscopy showed that the electron deposits in the thickened basement membrane were formed by randomly arranged 16- to 18-nm nonbranching fibrils. A Congo red stain for amyloid was negative. These findings corresponded with the diagnosis of fibrillary glomerulonephritis. Therefore, this case showed a rare combination of fibrillary glomerulonephritis and MGUS.

Aged↗
PubMed2006

Cystic beta2-microglobulin amyloidoma in a patient on long-term hemodialysis.

We report a patient with beta2 microglobulin amyloidosis (beta2M) in whom cystic tumors were seen in the bilateral axillary region. The patient was a 68-year-old woman who had been on hemodialysis for more than 20 years because of IgA nephropathy. Computed tomography-guided biopsy was performed to confirm the diagnosis. Congo red staining, beta2M immunohistochemistry, and electron microscopy examination of the biopsied sample showed extended beta2M deposits in the cystic tumor. beta2M-related amyloidosis in patients with long-term dialysis commonly presents as osteoarticular disease, although a soft-tissue pseudotumor, known as amyloidoma, has been reported. This is the first report in the English-language literature of amyloidosis presenting as bilateral axillary cystic tumors.

Aged↗
PubMed2005

Human immunoglobulin light chains lambda form amyloid fibrils and granular aggregates in solution.

Myeloma nephropathy is a disorder characterized by deposition of monoclonal immunoglobulin light chains in the kidneys. The chains deposited form either amyloid fibrils or granular (amorphous) aggregates. Distinct molecular mechanisms leading to the formation of different aggregate types in kidney of patients with multiple myeloma are poorly understood. Here we describe the self-association kinetics of human monoclonal immunoglobulin light chains lambda (GRY) isolated from urine of a patient with multiple myeloma. Under physiological conditions, the isolated light chain exists predominantly in a form of covalent dimer with apparent molecular mass of 50.1 kD. Spectral probe binding, analytical gel filtration, Western blot analysis, and electron microscopy indicate that GRY dimer aggregation occurs via two different pathways producing either amyloid fibrils or amorphous aggregates depending on microenvironment. Incubation of GRY (25 microM) for 4-14 days at 37 degrees C in phosphate buffered saline (PBS), pH 7.0, or in PBS containing urea (0.8 M), pH 6.5, leads to amyloid fibril formation. Under electron microscopy, the fibrils show unbranched thread-like structures, approximately 60-80 x 1000 A in size, which can bind thioflavin T and Congo Red. GRY maintained in acetate buffer, pH 3.5, forms granular aggregates. The structure of GRY oligomers formed during the early stage of amyloid fibril formation (1-4 days) has been examined by means of protein cross-linking with homobifunctional reagents. These oligomers are predominantly trimers and tetramers.

Amyloid↗
PubMed2006

Microscopic colitis in routine colonoscopies.

Lymphocytic colitis (LC) and collagenous colitis (CC), both known as microscopic colitis (MC), are uncommon entities with increasing incidence as more clinicians take biopsies from macroscopically normal colons and as pathologists use more rigorous diagnostic criteria to be confident of the diagnosis. Information on the incidence of this type of colitis is limited and based on reported cases. The purpose of this work is to estimate the incidence of LC and CC in reviewed routine colonoscopies. We reviewed 2815 colonoscopies performed at a tertiary referral center with an open-access service using restricted histological criteria in order to establish the frequency rate of LC and CC in routine colonoscopic biopsy material. Cases suspicious for MC were stained with Masson's trichrome or Congo red stain and immunohistochemically for lymphocytes, where appropriate. Review of routine colonoscopic biopsies showed that MC is underreported in our colonoscopic material. Incidence rates of LC and CC (0.9 and 0.4, respectively) were based on morphological assessment of colonoscopic biopsies using stringent criteria together with clinical data and after differentiation with other lesions which can mimic MC. The 10.2% rate of this type of colitis in patients with chronic watery diarrhea indicates the necessity to consider these lesions in older individuals with diarrhea and normal endoscopical colonic mucosa.

Adult↗
PubMed2007

Schistosoma-induced amyloidosis in hamsters is gender-dependent.

BACKGROUND: A high serum level of female protein (FP), found to be a constituent of Syrian hamster amyloid was associated with enhanced amyloidosis. In this work, we studied the sex-limited factors in the induction of amyloidosis in Syrian hamsters infected with either Schistosoma mansoni or S. hematobium cercariae. METHODS: Hamsters were infected with different species of schistosome cercariae and sacrificed after different time periods of infection. Kidney and liver specimens were processed in paraffin, stained with Congo-red and examined by ordinary light and polarized light microscopy. RESULTS: Statistical analysis showed a significant difference in intensity of kidney and liver amyloid deposits (P<0.002 & <0.007 respectively) between females and male hamsters with extensive deposits in the former. Amyloid deposits were correlated significantly to the duration of infection (P<0.001) than the load of worm recovered. CONCLUSION: From this study, we conclude that, in hamster model, Schistosoma-induced amyloidosis is enhanced in females than male hamsters. This may be due to the high serum level of FP that is normally detected in females. As an experimental model for schistosomal nephropathy, we recommend to use male hamsters instead of females to minimize the effect of amyloid deposits, which may mask other pathological changes associated with schistosomal infection.

Amyloidosis↗
PubMed2006

Nodular pulmonary amyloidosis.

A 72-year-old woman with a diagnosis of suspected rheumatoid arthritis was admitted with multiple pulmonary nodules in the bilateral lung field. To obtain a diagnosis, a nodule was resected using video-assisted thoracic surgery. Microscopically, amorphous eosinophilic acellular substances were surrounded by inflammatory infiltrates, which were confirmed to be amyloid deposits by congo red staining. Thus, a diagnosis of pulmonary amyloidosis was obtained. The clinical features and diagnostic process are discussed.

Aged↗
PubMed2006

Current treatment in cardiac amyloidosis.

Involvement of the heart is a common finding in amyloidosis. The heart is usually infiltrated by amyloid fibrils in primary amyloidosis and age-related forms of amyloidosis, less commonly in transthyretin familial amyloidosis, and rarely in secondary amyloidosis. The most common clinical presentation is restrictive cardiomyopathy with right-sided heart failure. The second most frequent presentation is congestive heart failure due to systolic dysfunction, followed by arrhythmias and orthostatic hypotension. The diagnosis of amyloidosis requires tissue sample confirmation; at present, Congo red staining in polarized light is the diagnostic method of choice. The characterization of protein fibril type by immunohistochemistry or biochemistry is essential for patient prognosis and treatment. The therapeutic approach consists of specific treatment of amyloidosis and supportive treatment for cardiac-related symptoms. The treatment depends on the type of amyloidosis and the stage of disease. The mainstay of supportive treatment of cardiac failure is diuretic therapy. Primary amyloidosis treatment protocol includes melphalan and prednisone chemotherapy. Heart transplantation is only a palliative treatment. Stem cell transplantation is an emerging treatment alternative. Combination therapy of melphalan and stem cell transplantation has been shown to be a promising treatment strategy. Secondary amyloidosis requires aggressive treatment of the associated inflammatory and neoplastic process. Age-related (senile) amyloidosis benefits from supportive cardiac treatment when applicable. Transthyretin amyloidosis, the most common cardiac hereditary amyloidosis, is treated by liver or combined liver-heart transplantation. New therapies based on chemical and immunologic reaction with amyloid or its precursor are under intensive development.

Journal Article↗
PubMed2002

Reduced beta-cell mass and expression of oxidative stress-related DNA damage in the islet of Japanese Type II diabetic patients.

AIMS/HYPOTHESIS: We examined the pancreatic islet lesions in Japanese patients with Type II diabetes mellitus to determine if the damage was related to oxidative stress. METHODS: Morphometric analyses were performed on immunostained sections of the tail portion of the pancreas from 14 diabetic and 15 non-diabetic patients. Amyloid deposition and oxidative stress-induced tissue damage were evaluated by Congo-red staining and immunostaining. Resistance to oxidative stress was assessed from immunostaining results for Cu, Zn-superoxide dismutase (SOD). Expression of (pro)insulin mRNA was assessed by in situ hybridisation. RESULTS: The pancreas from diabetic patients had amyloid deposition in about 15 % of the islets, intensified reactions of 8-OHdG and HNE, as well as reduced expression of SOD. Islet volume density of beta cells and total beta-cell mass in the pancreas from diabetic patients were reduced by 22 % (p < 0.001) and 30 % (p < 0.05). Islet volume density and total mass of (pro)insulin mRNA-positive cells were similarly reduced in diabetic patients by 22 % (p < 0.001) and 39 % (p < 0.05), respectively. Islet volume density of A cells was increased by 20 % (p < 0.001) but total mass did not change. There were no changes in volume densities of islet, D and PP cells. Reduced beta-cell volume density correlated with increased positive staining of 8-OHdG. CONCLUSION/INTERPRETATION: Japanese Type II diabetic patients show a reduction of beta-cell mass and evidence of increased oxidative stress-related tissue damage that is correlated with the extent of the beta-cell lesions.

Aged↗
PubMed1995

Cardiac immunocyte-derived (AL) amyloidosis: an endomyocardial biopsy study in 11 patients.

The objective of this study was to investigate the spectrum of morphologic features in myocardial biopsy specimens from patients with cardiac immunocyte-derived (AL) amyloidosis. Cardiac involvement is the most important predictor of survival in AL amyloidosis. Myocardial biopsy remains the method of choice for diagnosing cardiac amyloidosis when noninvasive studies give equivocal results. Histologic, immunohistochemical, ultrastructural, and morphometric studies were made on myocardial biopsy specimens from 11 patients in whom the diagnosis of AL amyloidosis was based on the demonstration of a monoclonal immunoglobulinopathy and of amyloid deposits in tissues. Histopathologic study showed amyloid in 10 of the 11 biopsies. In one biopsy (Congo red negative), the diagnosis was made by ultrastructural identification of amyloid fibrils. In all patients, the deposits formed perimyocytic layers that measured up to 18 microns in thickness. These layers formed along the basement membranes, which were partially preserved in 5 patients and unrecognizable in 6. Interstitial nodular deposits were also present in 5 patients. Immunohistochemical studies for the characterization of the proteins in the amyloid deposits were diagnostic in 1 patient and confirmatory in 10. Nodular deposits, thick perimyocytic layers of amyloid and small myocyte diameters were associated with shorter survival of the patients. Small-vessel involvement and myofilament loss occurred in all patients. In conclusion, myocardial biopsy serves to (1) establish the diagnosis of cardiac amyloidosis; (2) characterize immunohistochemically the proteins in the amyloid fibrils and (3) assess the degree of myocyte damage and atrophy.

Adult↗
PubMed1981

Amyloidosis in systemic lupus erythematosus.

Amyloidosis occurs in a significant proportion of patients with rheumatologic diseases. The fibrillar amyloid proteins in such patients are composed predominantly of amyloid A protein, which is characteristic of the amyloid deposits associated with chronic inflammatory diseases. Only four patients with amyloidosis associated with systemic lupus erythematosus (SLE) have been described previously; analyses of their fibrillar amyloid proteins were not reported. We present herein, a patient with SLE and amyloidosis. Histochemical staining of our patient's renal tissue with Congo red demonstrated that the amyloid deposits contained amyloid A protein, as defined by permanganate sensitivity. In addition, the patient's serum contained increased concentrations of serum amyloid A proteins. In review, each of the previously described patients with amyloidosis associated with SLE had renal amyloid deposits, with diagnosis in three during evaluation of proteinuria. Thus, although rare, amyloidosis should be considered in the differential diagnosis of proteinuria in patients with SLE.

Adult↗
PubMed1983

Frequency and distribution of senile cardiovascular amyloid. A clinicopathologic correlation.

Atrium, ventricle, aorta, lung, kidney, and rectum were removed at autopsy from 85 consecutive elderly patients (aged 80 years or older) and examined for amyloid with Congo red. All tissues containing amyloid were counterstained with an antiserum specific for amyloid fibril protein ASc1 and studied by immunofluorescence. Three distinct forms of amyloid were found: (1) all patients had senile aortic amyloid; (2) 78 percent of patients had isolated atrial amyloid; and (3) 25 percent of patients had senile cardiac amyloid of the ASc1 type. The cardiac amyloid deposits were small and widely scattered in more than 80 percent of patients with isolated atrial amyloid and in more than 50 percent of patients with ASc1-type amyloid. Of 21 patients with ASc1 amyloid, 19 had extracardiac involvement (lung in 81 percent of cases and rectum in 57 percent of cases). The kidney was not involved in any patient. The mean heart weight, frequency of atrial fibrillation, percentage of patients with heart failure, and frequency of myocardial infarction were increased in patients with cardiac amyloid, but these differences failed to reach statistical significance. There was no difference in the mean left ventricular wall thickness or degree of coronary atherosclerosis.

Aged↗
PubMed1984

Primary (AL) amyloidosis as a cause of breast masses.

Amyloidosis is rarely considered in the differential diagnosis of breast masses. During the past six years, 27 women with primary (24) and multiple myeloma-associated (three) amyloidosis (AL amyloid) were evaluated at our center. In five of these patients, amyloid was demonstrated on microscopic examination of breast tissue. The clinical presentations were similar to fibrocystic breast disease in two cases and malignancy in two others. Amyloidosis of the breast may be more common than previously recognized, especially considering the predilection of amyloid for depositing around fat cells. Therefore, pathologic examination of nonmalignant breast tissue should include Congo red staining and viewing under polarized light.

Amyloid↗